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We report the anesthetic management of Freeman-Sheldon (whistling face) syndrome in a two-month-old boy scheduled for lateral canthoplasty. He had features of the syndrome including blepharophimosis, hypertelorism, a flat nose, microstomia with a limited opening, micrognathia, a very short webbed neck, scoliosis and multiple arthrogryposis. He was fed with a naso-gastric tube and suffered from several episodes of aspiration and oxygen desaturation. Difficult airway and intubation were anticipated. Anesthesia was induced via a mask with sevoflurane, although mask ventilation was difficult. Direct laryngoscopy and the insertion of a laryngeal mask airway were impossible due to microstomia with the limited opening as anticipated. A naso-tracheal intubation was achieved using a fiberoptic bronchoscope via a fiberoptic mask while ventilating the lungs. The operation and anesthesia afterwards were uneventful. In the ward, he was given supplemental oxygen but with occasional desaturation episodes. Thirteen days after the operation he was found cyanotic and resuscitation was attempted but failed. Autopsy demonstrated the hypoplasia of the lungs and thorax, atelectasis and bronchitis.

The Freeman-Sheldon syndrome is a rare congenital myopathy and dysplasia that results in deformity of the face, hands, and feet. We describe here some problems in general anesthesia from the anesthetic management point of view. An 8-year-old girl with Freeman-Sheldon syndrome underwent surgery under general anesthesia for the correction of lip deformity and microstomia. Patients with this syndrome may present anesthetic problems involving difficulties in endotracheal intubation due to microstomia, micrognathia and neck rigidity, as well as postoperative respiratory complications and problems that relate to myogenetic morphology and myofunctional abnormalities. Preoperatively, we analyzed X-ray cephalograms to evaluate the difficulty of endotracheal intubation. Fiberscopic endotracheal intubation was performed; the time required was 55 minutes. On recovery from anesthesia and after becoming fully responsive, she was extubated in the operating room without any complications. Postoperative recovery was uneventful. Patients with Freeman-Sheldon syndrome should be managed by good preanesthetic preparation, and attention should be paid to postoperative respiratory complications due to the use of muscle relaxants and to securing intravenous access.